multidisciplinary approach to interstitial lung diseases
TRANSCRIPT
Multidisciplinary Approach to Interstitial Lung Diseases
RISHI RAJ
Case Study: Mr. D.S
Mr. D.S
• 64 YEAR OLD MALE
• SLOWLY PROGRESSIVE COUGH AND DYSPNEA OVER LAST FEW YEARS, MORE FOR
THE LAST ONE YEAR
• BASILAR CRACKLES ON EXAM
• CXR SHOWED INTERSTITIAL OPACITIES
• REFERRED TO YOUR CLINIC FOR A FORMAL EVALUATION
Broad/Simplistic categories of ILDs
Survival differs in ILDs
Treatment of non-IPF related ILDs
IMMUNOSUPPRESSIVE/CYTOTOXIC MEDICATIONS ARE USEFUL IN TREATING NON-IPF ILDS
INCLUDING CRYPTOGENIC ORGANIZING PNEUMONIA, HYPERSENSITIVITY PNEUMONITIS,
CONNECTIVE TISSUE ASSOCIATED ILD ETC.
Corticosteroids
Azathioprine
Mycophenolate
Cyclophosphamide
Others
Patients with IPF should generally not be treated chronically with corticosteroids
Patients with IPF on prednisone and azathioprine are more likely to die or be hospitalized than those on placebo
Nintedanib reduces rate of FVC decline in IPF patients
Pirfenidone reduces the rate of decline of FVC
Mr. D.S
• 64 YEAR OLD MALE
• SLOWLY PROGRESSIVE COUGH AND DYSPNEA OVER LAST FEW YEARS, MORE FOR
THE LAST ONE YEAR
• BASILAR CRACKLES ON EXAM
• CXR SHOWED INTERSTITIAL OPACITIES
• REFERRED TO YOUR CLINIC FOR A FORMAL EVALUATION
Clinical Context + HRCT pattern
Bronchoscopy, BAL and biopsy (in selected cases)
Treat, follow, and revisit diagnosis as necessary
Surgical Lung Biopsy
Multi-disciplinary discussion
Not Diagnostic
Not Diagnostic
Clinical exam is THE most important tool in the
diagnosis of Interstitial Lung Diseases
ILD from one etiology can present with different radiologic and histopathologic patterns
RADIOLOGIC PATTERNS HISTOPATHOLOGIC PATTERNS
ILDs from different etiologies share the same radiologic and histopathologic patterns
RADIOLOGIC PATTERNS HISTOPATHOLOGIC PATTERNS
ILD Questionnaires
Radiographs and other workup as indicated
SEROLOGIC TESTING
Rheumatoid factor
Anti-Scl 70
Etc.
FORMAL RHEUMATOLOGY CONSULTATION
Inhalational Exposures (Hypersensitivity Pneumonitis)
Medications and Occupations
OCCUPATIONAL LUNG DISEASES
Occupational history
ALL occupations
DRUGS
Common drugs
Nitrofurantoin
Methotrexate
Amiodarone
Etc.
Mr. D.S: Additional history
• SMOKED 1 PACK/DAY UNTIL 15 YEARS AGO
• INTERMITTENT WOODWORKING, BUT NOW WEARS MASK
• SOME MOLD IN BATHROOM IN HOME BUT OTHERWISE NO SIGNIFICANT MOLD
INFESTATION
• DOWN CLOTHING AND BEDDING AT HOME
• NO DIAGNOSIS OF A CONNECTIVE TISSUE DISEASE BUT COMPLAINTS OF JOINT PAIN IN
HANDS AND FEET WITHOUT ASSOCIATE SWELLING
• PHYSICAL EXAM DID NOT SHOW ANY EVIDENCE OF ACTIVE OR PAST CONNECTIVE
TISSUE DISEASE
Mr. D.S: Working Diagnostic Considerations
• IDIOPATHIC PULMONARY FIBROSIS
• CHRONIC HYPERSENSITIVITY PNEUMONITIS
• RHEUMATOID ARTHRITIS ASSOCIATED CONNECTIVE TISSUE DISEASE
22
Mr. D.S
• THE CRP, ESR, ANA, ANTI SCL 70, SSA, SSB, MYOSITIS PANEL WERE ALL
NEGATIVE EXCEPT FOR SLIGHT ELEVATION IN RF AND A POSITIVE ANTI CCP
Clinical Context + HRCT pattern
Bronchoscopy, BAL and biopsy (in selected cases)
Treat, follow, and revisit diagnosis as necessary
Surgical Lung Biopsy
Multi-disciplinary discussion
Not Diagnostic
Not Diagnostic
Clinical Context + HRCT pattern
Bronchoscopy, BAL and biopsy (in selected cases)
Treat, follow, and revisit diagnosis as necessary
Surgical Lung Biopsy
Multi-disciplinary discussion
Not Diagnostic
Not Diagnostic
HIGH RESOLUTION CT CHEST
VARIOUS PATTERNS
CT Chest: Usual Interstitial Pneumonia Pattern
CT Chest: Probable Usual Interstitial Pneumonia Pattern
CT Chest: Indeterminate Pattern
CT Chest: Alternative Diagnosis Pattern
HIGH RESOLUTION CT CHEST
INTERSTITIAL LUNG DISEASE PROTOCOL
Conventional vs High Resolution CT
CONVENTIONAL HIGH RESOLUTION CT
Prone vs. Supine Images
Mr. D.S: HRCT Images
Mr. D.S: HRCT Images
Mr. D.S: HRCT Images
Mr. D.S:
• CT CHEST SHOWED AN INDETERMINATE UIP PATTERN
• NOT HELPFUL IN NARROWING THE DIFFERENTIAL DIAGNOSIS
Mr. D.S: Working Diagnostic Considerations
• IDIOPATHIC PULMONARY FIBROSIS
• CHRONIC HYPERSENSITIVITY PNEUMONITIS
• RHEUMATOID ARTHRITIS ASSOCIATED CONNECTIVE TISSUE DISEASE
39
Mr. D.S:
• REFERRED TO RHEUMATOLOGY
• THE PATIENT DID NOT MEET CRITERIA FOR CONNECTIVE TISSUE DISEASE AND
RHEUM RECOMMENDED TO DIAGNOSE AND TREAT THE ILD AS IF IT WAS NOT RELATED
TO A CONNECTIVE TISSUE DISEASE
Clinical Context + HRCT pattern
Bronchoscopy, BAL and biopsy (in selected cases)
Treat, follow, and revisit diagnosis as necessary
Surgical Lung Biopsy
Multi-disciplinary discussion
Not Diagnostic
Not Diagnostic
Bronchoscopy, lavage and biopsy
Clinical Context + HRCT pattern
Bronchoscopy, BAL and biopsy (in selected cases)
Treat, follow, and revisit diagnosis as necessary
Surgical Lung Biopsy
Multi-disciplinary discussion
Not Diagnostic
Not Diagnostic
Surgical Lung BiopsyTHORACOSCOPIC (VATS) LUNG BIOPSY
3 incisions (5-10 mm)
Access to all aspect of the chest
Favored approach if patients will tolerate anesthesia
POSTOPERATIVE CARE
Chest tube in place (overnight
Majority are home in 1-2 days (>90% in our practice)
Primary concern is air leak
Mortality following surgical lung biopsy
Mr. D.S:
• REFERRED FOR A SURGICAL LUNG BIOPSY (VATS)
• UNEVENTFUL PROCEDURE AND RECOVERY
Mr. D.S: Surgical Lung Biopsy
Clinical Context + HRCT pattern
Bronchoscopy, BAL and biopsy (in selected cases)
Treat, follow, and revisit diagnosis as necessary
Surgical Lung Biopsy
Multi-disciplinary discussion
Not Diagnostic
Not Diagnostic
49
Agreement on the final diagnosis increases with multidisciplinary discussion
91 ILD PATIENTS
STEP 1 Expert clinicians and radiologists independently
reviewed HRCT
Opinion: Definite, probable, possible and not UIP
STEP 2 Clinicians and radiologists reviewed HRCT with clinical
information
No discussion between participants
STEP 3 Clinician and radiology conference; discussed results
with each other
STEP 4 Conference: Clinicians, radiologists and pathologists
discussing cases and their diagnoses
STEP 5 All discussants tried to reach a consensus diagnosis
Stanford Multidisciplinary Interstitial Lung Disease Conference
• PULMONARY MEDICINE
• THORACIC RADIOLOGY
• PULMONARY PATHOLOGY
• RHEUMATOLOGY
• LUNG TRANSPLANT
• THORACIC SURGERY
• CLINICAL RESEARCH
Mr. D.S: Final Diagnosis
CONSENSUS MULTIDISCIPLINARY DIAGNOSIS: IDIOPATHIC PULMONARY FIBROSIS
Clinical Context + HRCT pattern
Bronchoscopy, BAL and biopsy (in selected cases)
Treat, follow, and revisit diagnosis as necessary
Surgical Lung Biopsy
Multi-disciplinary discussion
Not Diagnostic
Not Diagnostic
Mr. D.S: Clinical Course
• STARTED ON ANTI-FIBROTIC MEDICATIONS
• TOLERATED WELL EXCEPT FOR MILD AND MANAGEABLE SYMPTOMS
Mr. D.S: Clinical Course
QUALIFIED FOR, AND ENROLLED IN A TRIAL FOR NOVEL THERAPEUTIC AGENT FOR
IDIOPATHIC PULMONARY FIBROSIS
Mr. D.S: Clinical Course
• STABLE FOR 3 YEARS, AND THEN PROGRESSED CLINICALLY
• RECEIVED A DOUBLE LUNG TRANSPLANT AND DOING WELL 1 YEAR
POSTOPERATIVELY
Questions